Dynamic MR lymphangiography for assessment of the central conducting lymphatics, including the retroperitoneal lymphatics, cisterna chyli, and thoracic duct, is performed by injecting gadolinium-based contrast material into the groin lymph nodes. The lymphatic system, an important component of the circulatory system with essential physiologic functions, can be affected by various disease processes. There has been a delay in the development of effective imaging methods for the lymphatic system due to its small size, which limits visualization as well as introduction of contrast material. Traditionally, the lymphatic system has been imaged by injecting contrast material or radiotracers into the feet or hands. This is not sufficient for assessment of the central conducting lymphatics (CCLs) (such as the thoracic duct or the cisterna chyli). Fluoroscopic intranodal lymphangiography with injection of oil-based contrast material into groin lymph nodes improves visualization of CCLs but is limited in practice owing to the use of radiation and the potential risk for paradoxical embolization in children with left-to-right shunt. Dynamic contrast material–enhanced (DCE) magnetic resonance (MR) lymphangiography, which is performed by injecting gadolinium-bas...

BACKGROUND Middle aortic syndrome (MAS) is a rare clinical entity in childhood, characterized by a severe narrowing of the distal thoracic and/or abdominal aorta, and associated with significant morbidity and mortality. MAS remains a relatively poorly defined disease. This paper systematically reviews the current knowledge on MAS with respect to etiology, clinical impact, and therapeutic options. METHODS A systematic search of 3 databases (Embase, MEDLINE, and Cochrane Central Register of Controlled Trials) yielded 1,252 abstracts that were screened based on eligibility criteria resulting in 184 full-text articles with 630 reported cases of childhood MAS. Data extracted included patient characteristics, clinical presentation, vascular phenotype, management, and outcomes. RESULTS Most cases of MAS are idiopathic (64%), 15% are associated with Mendelian disorders, and 17% are related to inflammatory diseases. Extra-aortic involvement including renal (70%), superior mesenteric (30%), and celiac (22%) arteries is common, especially among those with associated Mendelian disorders. Inferior mesenteric artery involvement is almost never reported. The majority of cases (72%) undergo endovascular or surgical management with residual hypertension reported in 34% of cases, requiring medication or reintervention. Clinical manifestations and extent of extra-aortic involvement are lacking. CONCLUSIONS MAS presents with significant involvement of visceral arteries with over two thirds of cases having renal artery stenosis, and one third with superior mesenteric artery stenosis. The extent of disease is worse among those with genetic and inflammatory conditions. Further studies are needed to better understand etiology, long-term effectiveness of treatment, and to determine the optimal management of this potentially devastating condition.

To evaluate the authors' 7-year experience with the percutaneous cecostomy procedure and the long-term outcome of the procedure. Since 1994, 163 tube cecostomies for fecal incontinence were performed in patients aged 2-23 years and who weighed 8-72 kg (mean, 32.2 kg). Underlying conditions included spina bifida (n = 106), imperforate anus (n = 53), Klippel-Feil deformity (n = 1), cerebral palsy (n = 1), Hirschsprung disease (n = 1), and paraplegia (n = 1). Ventriculoperitoneal shunts were present in 85 (52%) of the 163 patients. The authors have followed up 124 (76%) of the 163 cecostomy patients. Information regarding enema technique, satisfaction with the procedure, postprocedure problems, and long-term outcome of the procedure was obtained by interviewing either the patients or the parents. Tube placement was successful in all patients. One hundred ten (89%) of the 124 patients experienced a substantial decrease in the frequency of soiling accidents. The vast majority of patients expressed satisfaction with the procedure; 117 (94%) of the 124 patients rated the cecostomy procedure as better than the bowel control procedure used before. Late complications of the procedure included granulation tissue and accidentally dislodged tubes. Four patients elected to have their tubes removed for aesthetic and tube management reasons. There was no mortality related to the procedure, although one patient died of pneumonia 5 years later. The percutaneous cecostomy procedure is a safe and effective method for treating fecal incontinence.